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Hereditary neuropathy with liability to pressure palsy: fulminant development with axonal loss during military training

¹ Division of Neurology, University of Missouri School of Medicine, Columbia, MO, USA
² Department of Pathology, University of Missouri School of Medicine, Columbia, MO, USA

Correspondence to:
Correspondence to:
S H Horowitz MD
Division of Neurology, M-178, University of Missouri School of Medicine; Columbia, MO 65212, USA; horowitzs{at}health.missouri.edu

ABSTRACT
Hereditary neuropathy with liability to pressure palsy (HNPP) is characterised by recurrent mononeuropathies following minor trauma. We describe a case of fulminant HNPP beginning on the first day of military physical training. Protracted weakness, muscle atrophy, hand contractures, and multifocal sensory loss developed during a further three weeks of basic training. Nerve conduction changes were typical of HNPP, but without segmental slowing. Electromyographically, there was prominent acute denervation in muscles of the hands and right shoulder. Sural nerve biopsy demonstrated tomaculae and remyelination. Genetic testing revealed PMP-22 gene deletion. This case report demonstrates that HNPP can present with rapidly progressive peripheral nerve dysfunction and electrophysiological evidence of focal axonal loss.

Abbreviations: CMT1, Charcot–Marie–Tooth disease type 1; CV, conduction velocities; DML, distal motor latencies; DTR, deep tendon reflex; HNPP, hereditary neuropathy with liability to pressure palsy; MAP, motor action potential; PMP-22, peripheral myelin protein 22; SNAP, sensory nerve action potential

Keywords: hereditary neuropathy; pressure palsy; axonal loss